Next Article in Journal
Scrotal Lymphangiectasia with Penile Elephantiasis in Underlying Lymphatic Filariasis—Challenging the Diagnostic Mind! A Case Report
Previous Article in Journal
Type I Interferon Signature in Chilblain-Like Lesions Associated with the COVID-19 Pandemic
Open AccessCase Report

Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children

1
Department of Oral Pathology, Faculty of Dental Sciences, University of Peradeniya, Peradeniya 20400, Sri Lanka
2
Oral and Maxillofacial Surgery Unit, Base Hospital, Badulla 9000, Sri Lanka
3
Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, University of Peradeniya, Peradeniya 20400, Sri Lanka
4
Laboratory of Oral and Maxillofacial Pathology, Unit of Oral Medicine & Oral Maxillofacial Pathology, University Hospitals of Geneva and Faculty of Medicine, University of Geneva, 1211 GE 4 Geneva, Switzerland
*
Author to whom correspondence should be addressed.
Received: 16 November 2020 / Revised: 4 December 2020 / Accepted: 23 December 2020 / Published: 1 January 2021
Low-grade myofibroblastic sarcoma (LGMS) is a mesenchymal tumor of myofibroblasts that occurs more frequently in adults. A series of three cases is presented to illustrate that LGMS may also occur within the oral cavity in children and adolescents. The first case (Case 1) occurred intra-osseously in the mandible, while the remaining two presented as gingival swellings and were purely restricted to soft tissue (Cases 2 and 3). The intra-osseous lesion arose in a 7-year-old girl, whereas the gingival lesions were observed in a 12-year-old girl (Case 2) and a 13-year-old boy (Case 3). Histopathologically, all cases were composed of spindle shaped cells arranged into long fascicles showing mild to moderate degree of nuclear atypia. Ki-67 (MIB-1) proliferation activity was relatively low, amounting to 3–5% in all cases. Immunohistochemically, all cases showed smooth muscle actin (SMA) positivity in spindle cells, while desmin, beta catenin, cytokeratin, and CD34 were negative, resulting in a diagnosis of LGMS. In conclusion, current series of three cases of LGMSs that occurred in the oral cavity in a child and two adolescent patients is presented to highlight an emerging disease that requires additional data for further characterization. View Full-Text
Keywords: low-grade myofibroblastic sarcoma; oral cavity; mandible; gingiva; malignancy; immunohistochemistry low-grade myofibroblastic sarcoma; oral cavity; mandible; gingiva; malignancy; immunohistochemistry
Show Figures

Figure 1

MDPI and ACS Style

Jayasooriya, P.R.; Athukorala, C.; Attygalla, M.; Mendis, B.R.R.N.; Lombardi, T. Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children. Dermatopathology 2021, 8, 1-9. https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8010001

AMA Style

Jayasooriya PR, Athukorala C, Attygalla M, Mendis BRRN, Lombardi T. Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children. Dermatopathology. 2021; 8(1):1-9. https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8010001

Chicago/Turabian Style

Jayasooriya, Primali R.; Athukorala, Chamara; Attygalla, Manjula; Mendis, Balapuwaduge R.R.N.; Lombardi, Tommaso. 2021. "Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children" Dermatopathology 8, no. 1: 1-9. https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8010001

Find Other Styles

Article Access Map by Country/Region

1
Back to TopTop